Clinical features and major histocompatibility complex genes as potential susceptibility factors in pediatric immune thrombocytopenia
Journal
Journal of the Formosan Medical Association
Journal Volume
111
Journal Issue
7
Pages
370-379
Date Issued
2012
Author(s)
Abstract
Background/Purpose: Immune thrombocytopenia (ITP) is a heterogeneous autoimmune disorder with diverse response rates to treatments that include corticosteroids, intravenous immunoglobulins (IVIG), and splenectomy. The predisposing causes of this autoimmune disorder, one of which is immunogenetic susceptibility, have not been fully determined. We investigated whether clinical features and human leukocyte antigen (HLA) genotypes influence the occurrence, treatment response, and disease duration of childhood ITP in Taiwan. Methods: We performed HLA genotyping of 70 Taiwanese children with ITP and of 70 healthy controls and compared the data. Demographic data were also collected and evaluated. Results: The frequencies of heterozygous HLA-A11 and the HLA-Cw1 allele were both significantly decreased in the ITP group (p = 0.0160 and p = 0.0089, respectively), whereas the frequency of heterozygous HLA-DQ5 was significantly increased in the ITP group (p = 0.0057). Patients with HLA-DRB1*11 or -DRB1*15 were more likely to respond poorly to corticosteroids than IVIG (p = 0.0446 and p = 0.0008, respectively). In addition, we observed a positive association between HLA-A11 homozygosity and the development of persistent or chronic ITP [odds ratio (OR) = 6.3165, p = 0.0479]. The presence of HLA-DRB1*08 was, however, negatively correlated with the development of persistent or chronic ITP (OR = 0.1729, p = 0.0657). Children with antecedent of preceding illness (API) and with a younger age of onset were more likely to experience a better treatment response and shorter course of ITP. Conclusion: We suggest that API, age of onset, and particular HLA class I and class II alleles, may be involved in and influence the occurrence and disease duration of childhood ITP, as well as responses to different therapeutic approaches. ? 2012.
SDGs
Other Subjects
corticosteroid; HLA A antigen; HLA antigen; HLA C antigen; HLA DQ5 antigen; HLA DRB1 antigen; immunoglobulin; unclassified drug; article; autoimmune thrombocytopenia; child; controlled study; demography; disease course; disease duration; female; gene frequency; genetic susceptibility; genotype; heterozygote; homozygosity; human; major clinical study; major histocompatibility complex; male; preschool child; school child; Taiwan; treatment response; Alleles; Case-Control Studies; Child; Child, Preschool; Female; Gene Frequency; Genetic Predisposition to Disease; Genotyping Techniques; Heterozygote; HLA Antigens; Homozygote; Humans; Major Histocompatibility Complex; Male; Purpura, Thrombocytopenic, Idiopathic; Taiwan
Type
journal article